Neurofibromatosis is a neuroectodermal abnormality caused by dominant inheritance. Soft fibromas develop in children and are predominantly multiple hemispherical or tipped tumors of several millimeters to several centimeters in the trunk and proximal extremities, soft, dermal, pink or brown in color, increasing in size with age and developing more slowly in adulthood. The lesions of elephantiasis-like polyneurofibromas are often distributed along the nerve trunks and are multifocal, with soft nodules and plaques within and under the skin. About 10% of patients develop malignancy, especially in fast-growing, larger lesions, and the damage develops into neurofibrosarcoma. Coffee spots, commonly found on the trunk, especially on the lower back, are brown patches several centimeters in size, oval-shaped, with clear boundaries. A small number of patients develop papillomas in the mouth and giant tongues. Nearly half of them have mental retardation, intracranial tumors and seizures. The prognosis is poor in adults with rapid disease progression and large lesions due to malignancy. Nerve sheath tumors, also known as Chewang’s tumor, originate from the nerve sheath and are scattered soft masses. If the tumor involves nerve tissue, sensory disturbances may occur, especially pain and numbness in the corresponding area. Motor disorders are rarely seen, and at most, weakness is manifested in the affected area. This tumor is a benign lesion with slow growth and rarely recurs after surgical excision.